【小儿Icenko-Cushing病发病不典型的临床病例及简要文献回顾】。

Q4 Medicine Problemy endokrinologii Pub Date : 2022-05-14 DOI:10.14341/probl13102
M A Tiulpakov, O B Bezlepkina, E V Nagaeva, V N Azizian, A M Lapshina
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引用次数: 1

摘要

Itsenko-Cushing病是一种罕见的多系统疾病,其特征是由分泌acth的脑肿瘤引起内源性中枢高皮质醇血症。成人期Itsenko-Cushing病的发病率为每100万人中0.7-2.4例,所有病例中只有10%发生在儿童期。儿童发病年龄平均为12.0-14.8岁。该疾病在儿童中的典型表现,连同肥胖和动脉高血压,是生长速度下降。诊断中枢性高皮质醇症的金标准是脑MRI,然而,该方法在儿童中的有效性仅为50%。主要的治疗方法是神经外科经鼻经蝶窦切除垂体鞍内腺瘤,这使得超过65%的病例有可能达到缓解。本文描述了一个6.5岁儿童Itsenko-Cushing病的临床病例,伴有肥胖、动脉高血压、非典型“高”身材、平均速度和看不到的皮质瘤。本文介绍了诊断搜索的阶段,鉴别诊断和手术治疗的复杂性,治疗后随访的结果,并简要回顾了文献。
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[A clinical Case and brief literature review of Icenko-Cushing's Disease in a pediatric patient with atypical onset of the disease].

Itsenko-Cushing's disease is a rare, multisystem disease characterized by the presence of endogenous central hypercortisolism due to an ACTH-secreting brain tumor. The frequency of Itsenko-Cushing's disease in adulthood is 0.7-2.4 per 1 million population, and only 10% of all cases occur in childhood. The age of onset of the disease in children is on average 12.0-14.8 years. A typical manifestation of the disease in children, along with obesity and arterial hypertension, is a decrease in growth rates. The gold standard for diagnosing central hypercortisolism is MRI of the brain, however, the effectiveness of this method in children is only 50%. The main method of treatment is neurosurgical transnasal transsphenoidal removal of endosellar pituitary adenoma, which makes it possible to achieve remission in more than 65% of cases. This article describes a clinical case of Itsenko-Cushing's disease in a 6.5-year-old child with obesity, arterial hypertension, atypically «high» stature, average velocity and non-visualizable corticotropinoma. The article presents the stages of diagnostic search, the complexity of differential diagnosis and surgical treatment, the results of follow-up after the treatment and a brief review of the literature.

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来源期刊
Problemy endokrinologii
Problemy endokrinologii Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
1.40
自引率
0.00%
发文量
59
期刊介绍: Since 1955 the “Problems of Endocrinology” (or “Problemy Endocrinologii”) Journal publishes timely articles, balancing both clinical and experimental research, case reports, reviews and lectures on pressing problems of endocrinology. The Journal is aimed to the most topical issues of endocrinology: to chemical structure, biosynthesis and metabolism of hormones, the mechanism of their action at cellular and molecular level; pathogenesis and to clinic of the endocrine diseases, new methods of their diagnostics and treatment. The Journal: features original national and foreign research articles, reflecting world endocrinology development; issues thematic editions on specific areas; publishes chronicle of major international congress sessions and workshops on endocrinology, as well as state-of-the-art guidelines; is intended for scientists, endocrinologists diabetologists and specialists of allied trade, general practitioners, family physicians and pediatrics.
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